Management of Mast Cells in Interstitial and Peribronchial Lung Areas
For patients with mast cell infiltration in interstitial and peribronchial areas of the lungs, treatment with mast cell stabilizers such as cromolyn sodium is the most effective first-line therapy to prevent mediator release and associated symptoms. 1
Diagnosis and Assessment
Bronchoalveolar lavage (BAL) cellular analysis is recommended for patients with suspected interstitial lung disease to identify mast cell involvement, with BAL cell differential counts greater than 0.5% mast cells representing mastocytosis 2
Bone marrow biopsy analysis should be considered when systemic mastocytosis is suspected, with immunohistochemistry markers including mast cell tryptase, CD117, and CD25 to optimize quantification of mast cell burden 2
Evaluation for KIT D816V mutation using highly sensitive assays should be performed on peripheral blood and/or bone marrow when systemic mastocytosis is suspected 2
Treatment Approach
First-Line Therapies
Mast cell stabilizers (cromolyn sodium) should be administered to prevent mast cell degranulation by inhibiting the release of mediators from mast cells in the lungs 1
H1 antihistamines at higher doses (2-4 times FDA-approved doses) should be used to reduce inflammation and symptoms caused by histamine release from mast cells 3
Adding H2 receptor antagonists (such as famotidine) enhances control by blocking additional histamine pathways 3
Additional Pharmacologic Options
For patients with exercise-induced symptoms related to mast cell activation, pre-treatment with cromolyn sodium 10-15 minutes before exercise is recommended 1
Leukotriene modifiers can be used daily or intermittently to prevent mast cell-mediated symptoms without leading to tolerance 2
For patients with severe symptoms despite first-line therapy, inhaled corticosteroids should be added to decrease the frequency and severity of symptoms, though they may not eliminate all mast cell-related manifestations 2
In cases of nonasthmatic eosinophilic bronchitis with mast cell involvement that is persistently troublesome despite high-dose inhaled corticosteroids, oral corticosteroids should be considered 2
Management of Systemic Mastocytosis with Lung Involvement
For patients with advanced systemic mastocytosis affecting the lungs, midostaurin should be considered as it targets KIT mutations commonly found in systemic mastocytosis 2
Multidisciplinary management involving pulmonology, allergy/immunology, and hematology specialists is essential for comprehensive care 2, 3
Avoidance of Triggers
Identify and avoid specific triggers that cause mast cell activation, including environmental allergens, temperature extremes, and certain medications 3, 1
Caution should be exercised with opiates as they can trigger mast cell activation, though they should not be withheld if needed since pain itself can trigger mast cell degranulation 3
Monitoring and Follow-up
Regular monitoring of symptoms and lung function is necessary to assess treatment efficacy 2
For patients with systemic mastocytosis, serum tryptase levels should be monitored to assess disease activity 2, 3
Special Considerations
Perioperative management requires special attention in patients with mast cell disorders due to increased risk of anaphylaxis; pre-anesthetic treatment with anxiolytics, antihistamines, and possibly corticosteroids is recommended 2
During pregnancy, management should focus on symptom alleviation using medications that minimize potential harm to the fetus, with multidisciplinary involvement of high-risk obstetrics, anesthesia, and allergy specialists 2
Emerging Therapies
Novel treatments targeting mast cells, including monoclonal antibodies against IgE, IL-4/IL-13, IL-5/IL-5Rα, and TSLP, show promise for patients with severe disease 4, 5
Agonists of inhibitory receptors expressed by human mast cells (Siglec-8, Siglec-6) are under investigation for treatment of mast cell-related lung disease 4
By targeting mast cells in the interstitial and peribronchial areas of the lungs with appropriate medications and trigger avoidance, symptoms can be effectively controlled and disease progression potentially slowed.